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Blood, 1 July 2004, Vol. 104, No. 1, pp. 34-39. Prepublished online as a Blood First Edition Paper on February 26, 2004; DOI 10.1182/blood-2003-09-3167. This Article Full Text Full Text (PDF) All Versions of this Article: 2003-09-3167v1 104/1/34    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Cunningham, M. J. Articles by the Thalassemia Clinical Research Network, Search for Related Content PubMed PubMed Citation Articles by Cunningham, M. J. Articles by the Thalassemia Clinical Research Network, Related Collections Red Cells Free Research Articles Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS Complications of -thalassemia major in North America Melody J. Cunningham, Eric A. Macklin, Ellis J. Neufeld, Alan R. Cohen, and the Thalassemia Clinical Research Network From the Children's Hospital Boston, Dana Farber Cancer Institute, and Harvard Medical School, Boston, MA; New England Research Institutes, Watertown, MA; and Children's Hospital of Philadelphia, PA. Treatment of patients with -thalassemia major has improved dramatically during the past 40 years; however, the current clinical status of these patients remains poorly characterized. We performed a cross-sectional study of 342 patients in the Registry of the National Institutes of Health-sponsored Thalassemia Clinical Research Network. Evidence of hepatitis C exposure was present in 35% of tested patients, was associated with age, and had a rate of spontaneous viral clearance of 33%. Ferritin levels ranged from 147 to 11 010 ng/mL (median, 1696 ng/mL). Median hepatic iron content was 7.8 mg/g dry weight and 23% of patients had values of 15 mg/g dry weight or higher. No patients 15 years or younger and 5% of patients aged 16 to 24 years had heart disease requiring medication. Ten percent had cirrhosis on biopsy. Endocrinologic complications were common among adults. Seventy-four (22%) patients had recent implantable central venous access devices (CVADs) placed. Among 80 episodes of bacteremia in 38 patients, 90% were attributable to the CVAD. Among 330 patients who had received deferoxamine chelation therapy, 224 (68%) reported no complications. We conclude that hepatitis C, iron-related organ dysfunction, and complications of iron chelation therapy are strongly age-dependent in North American patients with -thalassemia. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: A. Roghi, S. Dellegrottaglie, P. Pedrotti, S. Pedretti, E. Cassinerio, and M. D. Cappellini Unexpected myocarditis in thalassaemia major patient screened for iron load cardiomyopathy BMJ Case Reports, February 16, 2009; 2009(feb04_1): bcr0820080811 - bcr0820080811. [Full Text] P. Harmatz, M. M. Jonas, J. L. Kwiatkowski, E. C. Wright, R. Fischer, E. Vichinsky, P. J. Giardina, E. J. Neufeld, J. Porter, N. Olivieri, et al. Safety and efficacy of pegylated interferon {alpha}-2a and ribavirin for the treatment of hepatitis C in patients with thalassemia Haematologica, August 1, 2008; 93(8): 1247 - 1251. [Abstract] [Full Text] [PDF] V. Di Marco, M. Capra, F. Gagliardotto, Z. Borsellino, D. Cabibi, F. Barbaria, D. Ferraro, L. Cuccia, G. B. Ruffo, F. Bronte, et al. Liver disease in chelated transfusion-dependent thalassemics: the role of iron overload and chronic hepatitis C Haematologica, August 1, 2008; 93(8): 1243 - 1246. [Abstract] [Full Text] [PDF] R. Setiabudy, P. A. Wahidiyat, and L. Setiawan Platelet Aggregation and Activation in Thalassemia Major Patients in Indonesia Clinical and Applied Thrombosis/Hemostasis, July 1, 2008; 14(3): 346 - 351. [Abstract] [PDF] E. Vichinsky Oral Iron Chelators and the Treatment of Iron Overload in Pediatric Patients With Chronic Anemia Pediatrics, June 1, 2008; 121(6): 1253 - 1256. [Full Text] [PDF] A. Banerjee, S. B. Chakrabarty, S. R. Karmakar, A. Chakrabarty, S. J. Biswas, S. Haque, D. Das, S. Paul, B. Mandal, B. Naoual, et al. Can Homeopathy Bring Additional Benefits to Thalassemic Patients on Hydroxyurea Therapy? Encouraging Results of a Preliminary Study Evid. Based Complement. Altern. Med., October 29, 2007; (2007) nem161v1. [Abstract] [Full Text] [PDF] A. Tefferi Iron Chelation Therapy for Myelodysplastic Syndrome: If and When Mayo Clin. Proc., February 1, 2006; 81(2): 197 - 198. [Full Text] [PDF] H.-y. Luo, J. Boudreaux, M. H. Steinberg, and D. H. K. Chui Patients with thalassemia in the United States Blood, June 15, 2005; 105(12): 4896 - 4897. [Full Text] [PDF] G. Papanikolaou, M. Tzilianos, J. I. Christakis, D. Bogdanos, K. Tsimirika, J. MacFarlane, Y. P. Goldberg, N. Sakellaropoulos, T. Ganz, and E. Nemeth Hepcidin in iron overload disorders Blood, May 15, 2005; 105(10): 4103 - 4105. [Abstract] [Full Text] [PDF] A. R. Cohen, R. Galanello, D. J. Pennell, M. J. Cunningham, and E. Vichinsky Thalassemia Hematology, January 1, 2004; 2004(1): 14 - 34. [Abstract] [Full Text] [PDF]

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